Vasculitis

Question 1

Heterogeneous group of diseases defined by the finding of inflammatory cells within the wall of the blood vessel

Answer 1

vasculitis

Question 2

vessel wall inflammation causes

Answer 2

occlusion of lumen leading to tissue ischemia

Question 3

a systemic necrotizing vasculitis of small and medium sized muscular arteries (occasionally large arteries) affecting nearly any organ system. Often associated with hepatitis B infection.

Answer 3

Classic polyarteritis nodosa (PAN) -

Question 4

a necrotizing granulomatous vasculitis affecting predominantly
the upper respiratory (nose, sinus, larynx)
lower respiratory (lungs)
kidney (renal pathology shows focal segmental necrotizing glomerulonephritis, often indistinguishable from that in PAN or idiopathic RPGN)

Answer 4

Granulomatosis with polyangiitis

Question 5

associated with cANCA (cytoplasmic ANCA) pattern and anti Proteinase 3 antibodies (PR3)

Answer 5

Granulomatosis with polyangiitis

Question 6

Associated with positive pANCA and anti myeloperoxidase antibodies (MPO), much less often cANCA

Lack of granulomatous inflammation is key
Less involvement of the upper respiratory tract

Answer 6

Microscopic Polyangiitis

Question 7

features eosinophils in the peripheral blood and in affected tissues.
Preceding history of progressively severe asthma
Predominance of lung involvement
Also associated with pANCA pattern but is a less strong association

Answer 7

Eosinophilic Granulomatis with Polyangiitis (Churg-Strauss)

Similar to Microscopic Polyangiitis

Question 8

Target antigen most often is proteinase 3

Correlates with Granulomatosis with Polyangiitis

Answer 8

cANCA= cytoplasmic ANCA

Question 9

Target antigen most often is myeloperoxidase

Correlates with microscopic PAN and Churg-Strauss

Answer 9

pANCA= perinuclear ANCA

Question 10

antibodies that target cytoplasmic components of neutrophils.

Answer 10

ANCA –

Question 11

disease of the elderly featuring involvement of new headache and threat of loss of vision.
Involves extracranial arteries off of the carotid but also the aorta itself and other large arteries off of the aorta.
Granulomas with giant cells seen in involved vessel walls in about 50% of cases

Answer 11

Giant cell (temporal) arteritis (GCA) –

Question 12

disease of aorta and large vessels off of aorta affecting predominantly young women. Can present as “pulseless disease” (eg: no pulses in arms).
Pathologically indistinguishable from Giant Cell Arteritis but is less likely to involve extracranial arteries off of the carotid than GCA

Answer 12

Takayasu arteritis

Question 13

A predominantly small blood vessel vasculitis thought due to hypersensitivity reaction to a foreign antigen

Answer 13

Hypersensitivity vasculitis group

Question 14

small vessel vasculitis featuring presence of cryoglobulins in blood and involved blood vessel walls

strong HepC association

Answer 14

Cryoglobulinemic vasculitis –

Question 15

Vasculitis secondary to known primary diseases: (3)

Answer 15

1. connective tissue diseases (eg lupus)
2. malignancy
3. infection (eg HIV)

Question 16

acute syndrome in children
Mucosa (lips, tongue), skin and blood vessels show inflammation
Dreaded danger is involvement of coronary arteries with potential aneurysm formation and coronary occlusion

Answer 16

kawasaki

Question 17

treatments for GPA

Answer 17

1. cyclophosphamide ( can develop pancytopenia)
2. rituximab
3. azathioprine